Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Collecting duct carcinoma of the kidney is a rare and aggressive neoplasm of the distal collecting tubules for which there is no established therapy. We describe a young woman with metastatic collecting duct carcinoma who responded to Taxol/carboplatin chemotherapy with an 80% reduction in her tumor burden, including complete regression of lymph node metastases and significant shrinkage of a renal mass. She was rendered free of disease through nephrectomy and has been without a recurrence for 20 months. This suggests that Taxol/carboplatin chemotherapy and surgery should be considered for the treatment of metastatic collecting duct carcinoma.
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PMID:Long-term remission in a patient with metastatic collecting duct carcinoma treated with taxol/carboplatin and surgery. 1174 92

Renal medullary carcinoma is a recently recognized epithelial malignant tumor arising in the renal parenchyma. The tumor is almost exclusive to young black patients with the sickle cell trait. Most patients present with metastatic disease and have a poor prognosis. An Hispanic woman with renal medullary carcinoma who initially responded to chemotherapy with methotrexate, vinblastine, doxorubicin, and cisplatin and survived for 12 months is presented. The clinical, histologic, and radiologic features of this tumor are described, and chemotherapeutic regimens used in this disease are detailed. Treatment modalities have proved largely unsuccessful in the setting of advanced disease. Given the shared demographic, clinical, and radiographic features of these patients, awareness and early diagnosis may prove essential in improving survival.
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PMID:Renal medullary carcinoma: case report and review of the literature. 1452 77

Renal medullary carcinoma (RMC), an aggressive malignant epithelial neoplasm, first emerged as a distinct clinicopathologic entity in 1995. It affects individuals 40 years of age or younger and is strongly associated with sickle cell disease or trait. The majority of patients with RMC have widely disseminated disease at the time of diagnosis and most fail to respond to both chemotherapy and radiotherapy. Mortality approaches 100%, and death usually occurs within a few months to a year of diagnosis. We report a 6-year-old African-American boy with a history of gross hematuria who died four weeks after diagnosis of disseminated metastatic disease. Autopsy showed a 4.4-cm renal mass with metastases to the contra lateral kidney, liver, lungs and multiregional lymph nodes. RMC should be included in the differential diagnosis of any patient 40 years old or younger with a history of hemoglobinopathy and gross hematuria and/or abdominal or flank pain. A brief discussion of the differential diagnosis, histogenesis and treatment is presented in this study.
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PMID:Renal medullary carcinoma in a six-year-old boy with sickle cell trait. 1453 Aug 15

Renal medullary carcinoma is an epithelial malignant tumor arising from collecting duct epithelium. The tumor is almost exclusive to young black patients with the sickle cell hemoglobinopathies, mainly sickle cell trait (SCT). Most patients present with metastatic disease and have a worse prognosis. An African-American male with sickle cell disease (HbSCD) who was diagnosed to have renal medullary carcinoma is presented here. The clinical, histologic and radiologic features of this tumor are described. In the setting of advanced disease, treatment modalities have proved largely unsuccessful. Given the shared demographic, clinical and radiographic features of these patients, awareness and early diagnosis may prove essential in improving survival.
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PMID:Renal medullary carcinoma. 1689 89

Renal medullary carcinoma is a rare, well-recognized highly aggressive tumor of varied histopathology, which occurs in young patients with sickle cell trait or disease. Rhabdoid elements, occasionally seen in high-grade renal tumors including renal medullary carcinoma, possibly represent a pathologic marker of aggressive behavior. INI1 (hSNF5/SMARCB1/BAF47) is a highly conserved factor in the ATP-dependent chromatin-modifying complex. Loss of this factor in mice results in aggressive rhabdoid tumors or lymphomas. In humans, the loss of INI1 expression has been reported in pediatric renal rhabdoid tumors, central nervous system atypical teratoid/rhabdoid tumors and epithelioid sarcomas, a possible primary soft tissue rhabdoid tumor. This study compares five renal medullary carcinomas with 10 high-grade renal cell carcinomas (five with rhabdoid features), two urothelial carcinomas and two pediatric renal rhabdoid tumors. All five renal medullary carcinomas, irrespective of histopathology, showed complete loss of INI1 expression similar to that seen in pediatric renal rhabdoid tumors. In contrast, all renal cell carcinomas or urothelial carcinomas, including those with histological rhabdoid features, expressed INI1. Clinically, all five of the patients with renal medullary carcinoma and the two patients with rhabdoid tumors presented with extra-renal metastases at the time of diagnosis. This study demonstrates that renal medullary carcinoma and renal rhabdoid tumor share a common molecular/genetic alteration, which is closely linked to their aggressive biological behavior. However, the absence of INI1 expression is not necessarily predictive of rhabdoid histopathology but remains associated with aggressive behavior in renal medullary carcinoma.
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PMID:Renal medullary carcinoma: rhabdoid features and the absence of INI1 expression as markers of aggressive behavior. 1832 9

Collecting duct carcinoma (CDC) of the kidney is a rare and aggressive malignant tumor arising from the distal collecting tubules which has been shown to have a poor response to several kinds of systemic therapy. We present a case of metastatic CDC that responded favorably to a multiple tyrosine kinase inhibitor, sunitinib, achieving a partial response in both lung and skeletal metastases. To our knowledge, this is the first report showing therapeutic activity of sunitinib against CDC. Considering these findings, it would be worthwhile prospectively investigating the role of multiple tyrosine kinase inhibitors, particularly sunitinib, in the management of metastatic CDC.
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PMID:Metastatic collecting duct carcinoma of the kidney responded to sunitinib. 2068 10

A 40-year-old woman presented to the Brooke Army Medical Center Emergency Department complaining of 2 episodes of gross hematuria. Computed tomography and intravenous pyelogram revealed a right renal mass. A radical nephrectomy was performed with complete excision of the mass with negative margins on histological examination. The patient was diagnosed with renal medullary carcinoma, Fuhrman grade 4, based upon histological examination. A positron emission tomography scan revealed no other evidence of the disease. The pathologic stage was stage I renal medullary carcinoma. Four months after her nephrectomy, the patient developed a papule on her right frontal scalp. Initially thought to be a cyst, the papule increased in size over the course of 2 months and eventually ulcerated. At that time, she presented to the Wilford Hall Medical Center Dermatology Clinic with a 2.5-cm ulcerated tumor with pink rolled borders. A punch biopsy of the tumor revealed an infiltrating carcinoma with scattered glandular lumina and desmoplastic and mucinous stroma. The carcinoma was completely intradermal. Expert consultation confirmed the diagnosis of metastatic renal medullary carcinoma. Clear cell (conventional) and papillary renal cell carcinomas are known to metastasize to the skin, including the scalp. Renal medullary carcinoma commonly metastasizes to the regional lymph nodes, lung, liver, or adrenals. To our knowledge, this is the first report of a cutaneous metastasis of renal medullary carcinoma.
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PMID:Renal medullary carcinoma metastatic to the scalp. 2191 32

Collecting duct carcinoma (CDC) of the kidney is a rare and aggressive malignant tumor arising from the distal collecting tubules which has been shown to have a poor response to several kinds of systemic therapy. We present a case of metastatic CDC that responded favorably to a multiple tyrosine kinase inhibitor, sunitinib, achieving a partial response in both lung and skeletal metastases. To our knowledge, this is the first report showing therapeutic activity of sunitinib against CDC. Considering these findings, it would be worthwhile prospectively investigating the role of multiple tyrosine kinase inhibitors, particularly sunitinib, in the management of metastatic CDC.
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PMID:Metastatic collecting duct carcinoma of the kidney treated with sunitinib. 2202 97

Renal medullary carcinoma (RMC) is a rare and highly aggressive malignancy arising from the renal medulla and found mostly in patients with sickle cell trait. RMC usually presents with widely metastatic disease. We describe a young man diagnosed with metastatic RMC who sustained a complete response to systemic chemotherapy but developed brain metastases with leptomeningeal involvement and subsequently had a partial response to brain irradiation. The use of radiation in the management of RMC is reviewed. Due to the apparent propensity for RMC to spread to the central nervous system, prophylactic treatment such as craniospinal irradiation should be considered along with chemotherapy in patients with metastatic RMC to potentially improve the progression-free interval.
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PMID:Response to radiation in renal medullary carcinoma. 2206 39

Collecting duct carcinoma (CDC) is a relatively rare subtype of renal cell carcinoma (RCC) which has an aggressive course with an extremely poor prognosis. Here we report on a case of CDC in a 29-year-old woman who showed rapid disease progression with some uncommon clinical features including extensive vertebral metastases and invasion of the spinal meninges. The patient developed paraplegia and died 9 months after the diagnosis of CDC. The features of the fulminant clinical course with the lesions of the meninges, although rare, are important for the accumulation of experience of this rare disease.
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PMID:Collecting duct carcinoma presenting uncommon metastatic features. 2323 69


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